Porphyria, Sun Exposure Skin Blisters

Porphyria is a disorder that resulted in the accumulation of chemicals called porphyrins within the body. Porphyrin is actually a normal body chemicals, but not normal if the number had grown a lot.

The cause is usually an inherited gene mutation, but environmental factors can trigger the development of symptoms in some types of porphyria. Porphyria usually affects the nervous system, skin or both. Specific signs and symptoms of porphyria depends on the genes that did not norm

symptom

There are two general categories namely porifiria acute porphyria and porifiria skin.

Acute porphyria. Porifiria disease causes symptoms in the nervous system and skin. Acute porphyria attacks rarely occur before puberty and after menopause in women. Signs and symptoms may last from one to two weeks.
Possible signs and symptoms include:
1. insomnia
2. Anxiety or restlessness
3. Severe abdominal pain
4. constipation
5. gag
6. diarrhea
7. Pain in legs, arms, or back
8. Muscle pain, tingling, numbness, weakness or paralysis
9. dehydration
10. excessive sweating
11. convulsions
12. confusion
13. hallucinations
14. disorientation
15. paranoia
16. Red colored urine
17. High blood pressure

Skin porphyria. Porifiria skin diseases cause symptoms of skin is too sensitive to sunlight, but does not affect the nervous system. Some forms of porphyria skin begins to show signs and symptoms when the infant or childhood, namely:
1. itch
2. Pain and redness (erythema)
3. Skin swelling (edema)
4. blister
5. Red colored urine

Cause

Porphyria arise from impaired production of substances in the body called heme. Heme is found in all tissues, most commonly found in red blood cells, bone marrow and liver. Heme is a major component of hemoglobin, iron-rich protein that gives red color to blood. Hemoglobin enables red blood cells carry oxygen from the lungs to all parts of the body and carry carbon dioxide from other parts of the body to the lungs so it can be released when exhaling.

There are eight enzymes that convert chemicals called porphyrins into heme. In porphyria, inherited mutation of one gene involved in heme production can cause the enzyme deficiency, which can cause porphyrin accumulate in the body. Although porphyrins are normal body chemicals, are not normal if the number had grown a lot.

Most porphyrias are inherited. Some forms of this disease is caused by defective genes inherited from one parent, some other forms derived from both parents. This gene defect causes one or more enzymes involved in the process of conversion of porphyrins into heme is not running normally.

A person who inherits the gene causes porphyria does not mean that he will show signs and symptoms. He may have latent porphyria and never have any signs and symptoms. But this latent porifiria will be the operator for most of the abnormal gene.

Environmental factors may trigger the development of signs and symptoms in some types of porphyria. When exposed to the trigger, the body's demand for increased production of heme. This will master the enzyme deficiency and cause signs and symptoms porifiria.
Triggers include:
1. Drugs (most often barbiturates and sulfonamide antibiotics. Birth control pills and sedatives can also cause symptoms)
2. Diet or fasting
3. Smoke
4. Infection
5. Stress
6. Use of alcohol
7. Menstruation
8. Exposure to sunlight
9. Excess iron in the body

Treatment

Treatment of acute porphyria focus on relieving symptoms. May require hospitalization for severe cases. Treatment includes:
1. Stopping the drug may have triggered the symptoms
2. Drugs to control pain
3. Providing treatment of infections or other diseases that may cause symptoms
4. Infusion of sugar (glucose) to maintain carbohydrate intake
5. Intravenous fluids to combat dehydration
6. Hemin injection or hePorfiria, Sun Exposure Skin Blisters

Treatment of skin porphyria focuses on reducing the number of porphyrins in the body and to help relieve symptoms, include:
1. Expenditure of blood to reduce iron in the body resulting in lower levels of porphyrins. May need to undergo several processes before entering the stage of extravasation of healing.

2. Drugs. Drugs commonly used to treat malaria; hydroxychloroquine (Plaquenil) and chloroquine (Aralen), can absorb excess porphyrins help rid the body more quickly. These drugs are generally used only in people who can not tolerate phlebotomy.

3. Beta carotene. This is for long-term treatment. The body converts beta carotene into vitamin A which is needed for healthy eyes and skin. Beta carotene may increase skin tolerance to sunlight.